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Magill’s Medical Guide, 9th Edition

Sturge-Weber syndrome

by Elizabeth D. Schafer, PhD

Category: Disease/Disorder

Also known as: Sturge-Weber-Dimitri syndrome, encephe-lotrigeminal angiomatosis

Anatomy or system affected: Blood, blood vessels, brain, circulatory system, eyes, head, nerves, nervous system, skin

Specialties and related fields: Dermatology, neurology, ophthalmology, pediatrics, plastic surgery, psychology, vascular medicine

Definition: A disorder associated with partial facial disfigurement which involves vascular accumulations that affect the central nervous system.

CAUSES AND SYMPTOMS

Although the precise causation of Sturge-Weber syndrome has not been established, medical researchers have found a relationship to excess circulatory vessels remaining from embryonic development. Professionals estimate that approximately 15 percent of newborns with port-wine stains have this syndrome. Patients usually exhibit a facial port-wine stain along the trigeminal nerve on one side. The size of the birthmark and the area covered depend on which, and how many, of the three branches of this nerve-found in the brow, cheek, and jaw-are affected by excessive capillaries delivering blood to the skin. Sometimes, the birthmark appears on other parts of the body or the entire head. Infrequently, patients with this syndrome do not have a port-wine stain.

Neurological activity is often disrupted and slowed in these patients because of the massing of circulatory vessels on the brain adjacent to the port-wine stain. As a result, patients sometimes experience calcium buildup in brain tissues and epileptic seizures on the side without the birthmark. Approximately half of patients suffer developmental problems, including intellectual disabilities. Because circulation to the eyes is altered, fluid drainage is sometimes disrupted and pressure intensifies, resulting in swelling, glaucoma, and possible blindness. Partial paralysis occasionally accompanies this syndrome.

TREATMENT AND THERAPY

Many patients with Sturge-Weber syndrome suffer only facial birthmarks and mild neurological symptoms that do not affect their life span. Lasers can erase port-wine stains, and cosmetics are a less expensive but temporary solution. Psychological counseling can help patients cope with societal reaction to their birthmarks, including bullying and rejection.

Patients whose brains are significantly affected have higher risks. Medications can treat convulsions. In extreme cases, surgery may be required to remove damaged tissues. Medical professionals X-ray patients to assess brain calcification and to locate any tumors. The eyes are examined regularly to detect problems and are treated with medication or surgery as applicable. Physical therapy can strengthen weakened muscles in cases of partial paralysis.

Information on Sturge-Weber Syndrome

Causes: Unknown; possibly excess blood vessels remaining from embryonic development

Symptoms: Port-wine stain, seizures, intellectual disabilities, eye disorders (swelling, glaucoma, possible blindness), sometimes partial paralysis

Duration: Chronic

Treatments: Depends on severity; may include cosmetic correction, including laser removal), psychological counseling, anticonvulsive medications, surgery to remove damaged tissues

PERSPECTIVE AND PROSPECTS

William Allen Sturge hypothesized in 1879 that a six-year-old patient suffering from seizures had a brain lesion on the same side as her port-wine stain. His peers rejected his theory. By 1901, Siegfried Kalischer verified Sturge’s hypothesis through pathological studies. In 1922, Frederick Parkes Weber used radiography to document calcification inside the craniums of patients with port-wine stains. The next year, Vincente Dimitri added his insights; he is sometimes incorporated into the name of this syndrome. E. Steve Roach described three types of Sturge-Weber syndrome in 1992. His categories are based on trait occurrence and extent.

Established in 1987, the Sturge-Weber Foundation has created a registry of individuals with this syndrome. In the early twenty-first century, researchers continued their investigations of possible genetic factors in this syndrome.

See also Birthmarks; Blood vessels; Brain damage; Circulation; Congenital disorders; Dermatology; Dermatology, pediatric; Eyes; Glaucoma; Nervous system; Neuralgia, neuritis, and neuropathy; Neurology; Neurology, pediatric; Paralysis; Plastic surgery; Seizures; Skin; Skin disorders; Vascular medicine; Vascular system.

For Further Information:

1 

Ball, Karen Fisher, ed. Sturge-Weber Syndrome: The Resource Guide for a Reason, a Season, and a Lifetime. Mt. Freedom, N.J.: Sturge-Weber Foundation, 2003.

2 

Bodensteiner, John B., and E. Steve Roach, eds. Sturge-Weber Syndrome. Mt. Freedom, N.J.: Sturge-Weber Foundation, 1999.

3 

Guttman, Cheryl. “Location of Port-Wine Stains May Signal Syndromal Associations.” Dermatology Times 19, no. 7 (July, 1998): 25.

4 

Sturge-Weber Foundation. www.sturge-weber.org.

Citation Types

Type
Format
MLA 9th
Schafer, Elizabeth D. "Sturge-Weber Syndrome." Magill’s Medical Guide, 9th Edition, edited by Anubhav Agarwal,, Salem Press, 2022. Salem Online, online.salempress.com/articleDetails.do?articleName=MMG2022_1300.
APA 7th
Schafer, E. D. (2022). Sturge-Weber syndrome. In A. Agarwal, (Ed.), Magill’s Medical Guide, 9th Edition. Salem Press. online.salempress.com.
CMOS 17th
Schafer, Elizabeth D. "Sturge-Weber Syndrome." Edited by Anubhav Agarwal,. Magill’s Medical Guide, 9th Edition. Hackensack: Salem Press, 2022. Accessed September 16, 2025. online.salempress.com.